AB0642 Diagnosis of Cerebral vasculitis: retrospective study of 68 cases
Authors: A. Ben Salem, S. Arfa, F. Sghaier, H. Bouguila, Z. Chaari, A. Bchir, S. Younes, O. Berriche
Journal: Annals of the Rheumatic Diseases (2022)

Background Cerebral vasculitis, whether primary or secondary, is a group of heterogeneous pathologies characterized by a destructive inflammatory vascular wall lesion of the cerebral tissue. Its physiopathology remains poorly understood. Clinical polymorphism is a major characteristic of this disease. Objectives The aim of this work was to describe the epidemiological, clinical, radiological and etiological aspects of Cerebral vasculitis. Methods We conducted a retrospective descriptive study based of the records of 68 patients who were hospitalized in the Internal Medicine and Neurology Departments of the Taher Sfar University Hospital in Mahdia over a 12-year period from January 2008 to June 2021. Results A total of 68 patients were included: 46 female and 22 male (sex-ratio M/F: 0.47) with an average age of 50.8 ±12.7 [15-75 years]. The revealing symptoms were: Headaches in 52 patients (79.9%) followed by motor deficit in 21 patients (30.8%), sensitivity disorder in 24 patients (35.3%) and neurosensory disorders in 24 patients (35.3 %). These symptoms were insidious in 41 patients (61.2%). The neurological examination was pathological in 75 % of the patients, among them 19 patients (28.7%) had a walking disorder. Strokes were the revealing complication in 5 patients (7%). Laboratory tests revealed an inflammatory syndrome in 37 patients (55.2%). The lumbar puncture was performed in 76.5% of cases. Cerebral spinal fluid showed aseptic meningitis in 5 cases and High protein level in 6 patients. The immunologic investigations showed that Antinuclear antibodies (ANA) were positive in 41 % and antiphospholipid antibodies were positive in 7.3%. Cerebral Magnetic Resonance Imagining (MRI) revealed multiple patchy lesions hyperintense on T2 and FLAIR in the white matter in all cases: bilateral and asymmetric in 55 patients (80.9%) with subcortical distribution in 63% and periventricular localization in 45 %. Three patients had Leptomeningeal enhancement. The etiologies of cerebral vasculitis were: Sjogren Syndrome in 33.8 %, Systemic lupus Erythematosus in 22%, Behcet disease in 19.1 %, Giant cell arteritis in 7.3%, antiphospholipid syndrome in 5.8%, Sarcoidosis in 4.4 %, Susac syndrome in 4.4 % and systemic sclerosis in 2.9 %. Treatment was based on oral corticosteroids in 89.7 % of cases, associated with intravenous corticosteroids in 52.9% of cases. Immunosuppressive therapy was indicated in 42.4% of patients. Prompt improvement was obtained in 48,3%, symptoms stability was noticed in 20 %. However, 30% of patients experienced relapse. Worsening of signs was seen in 1.7 %. Conclusion Cerebral vasculitis is a rare disorder with a non-specific clinical presentation. Therefore, its diagnosis is a challenge for clinicians who base their investigations on clinical and paraclinical arguments to attribute the neurological and systemic events to vasculitis. An early diagnosis and an appropriate treatment could improve its prognosis. Disclosure of Interests None declared